Eur Arch Otorhinolaryngol DOI 10.1007/s00405-016-4087-1
CASE REPORT
Vascular leiomyoma of the nasal cavity: case report and literature review Thomas Mathieu1 • Annelies Verbruggen1,2 • Gerda Goovaerts3 • Frank Declau1,2
Received: 30 January 2016 / Accepted: 5 May 2016 Ó Springer-Verlag Berlin Heidelberg 2016
Abstract We report a case of a 54-year-old man with an angioleiomyoma originating from the right nasal floor. Nasal vascular leiomyomas are extremely rare tumours. A review of the literature revealed a limited number of cases of vascular leiomyoma in the nasal cavity. Clinically, they are characterised by nasal obstruction, epistaxis or pain as the primary symptom. Up to our knowledge, this is the first case in which a 3D CT scan was performed preoperatively. 3D CT scan reconstructions can show the delineation of the tumour very accurately helping to establish the therapeutic plan for removal. The definite diagnosis can only be confirmed by histopathology. Histopathologically, a vascular leiomyoma demonstrates proliferation of smooth muscle cells intermingled with dilated venous vessels. Keywords Vascular leiomyoma Nasal cavity Angioleiomyoma Smooth muscle Myopericytoma
Introduction Vascular leiomyomas or angioleiomyomas are benign tumours of myogenic origin. These tumours occur commonly in the uterus but are rarely found in the nasal cavity or the paranasal sinuses because of the paucity of smooth
muscle cells in the nose. Stout et al. first described this kind of tumour in 1937 [1]. In a localisation study of 7748 cases by Enzinger and Weiss [2], 95 % of the tumours presented in the female genitalia, 3 % in the skin, 0.9 % in the gastrointestinal tract, and the remainder occurred from various sites. Maeseka et al. described the first case of a nasal vascular leiomyoma [3]. Since then, approximately 32 cases of vascular leiomyomas in the nasal cavity have been described. They make up less than 1 % of all leiomyomas in the human body. Vascular leiomyomas have also been reported in other locations in the head and neck area. There are published cases in various anatomical sites such as larynx, auricle, oral cavity, tongue, tonsil, lip, cheek, trachea, oesophagus, submandibular gland and parotid gland and orbit [4–6]. Besides the nasal cavity, the ears and the lips are the most common sites of occurrence. Nearly all of the tumours developed in the skin or in the mucosa such as the oral or nasal mucosa. Involvement of the skull base is very rare [4, 6–8]. In contrast to nasal tumours, almost all vascular angioleiomyomas in the head and neck region are asymptomatic. Pain that is the most characteristic subjective symptom in patients with angioleiomyoma in other anatomical regions does rarely occur in the head and neck angioleiomyomas [4–6].
Patient report & Frank Declau
[email protected] 1
Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, Belgium
2
Department of ENT, GZA Hospitals, Campus St-Vincentius, St-Vincentiusstraat 20, 2018 Antwerp, Belgium
3
Department of Anatomopathology, GZA Hospitals, Antwerp, Belgium
A Caucasian male, 54 years old, visited our Otorhinolaryngology Department in October 2015. He presented with a 2-year history of a progressively growing tumour in the right nasal cavity. The patient sometimes had a feeling of nasal obstruction and he suffered recurrent small volume epistaxis episodes, which were difficult to stop. He was also complaining about pain at the level of the maxillary sinuses. Twenty years ago, the patient had a septoplasty
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because of a deviated septum. The patient has a known allergy to dust mites and grass pollens. Otorhinolaryngologic examination and fiberendoscopy were uneventful except for a submucous swelling at the right nasal floor leading to partial obstruction of the right nostril. There were also secretions at the bottom of the nasal passage at the right side. There were no pathological lymph nodes. A high resolution CT scan (Siemens HealthcareÒ) of the nose and sinuses showed a homogeneous and isodense tumour at the right nasal floor (2 9 1 cm) with opacification of the right maxillary sinus. Also a 3D-CT reconstruction of the skull was created (OsirixÒ, Switzerland). These images showed a deformation of the right nasal floor at the anterior piriform aperture: an impression of the right processus alveolaris of the maxillary bone at the level of the tumour was seen with a regular cortical delineation demonstrating bone remodelling (Fig. 1). According to the anatomic delineation of the tumour on CT imaging, the patient underwent a surgical exploration through a subgingival incision. The tumour was completely excised with a surrounding rim of normal nasal mucosa. Because of the presence of chronic rhinosinusitis, the patient also underwent a functional endoscopic sinus surgery during the same operation. Postoperative recovery was uneventful. The patient has shown no recurrence of the tumour up to now. The epistaxis and nasal obstruction resolved completely.
Pathological findings The tumour consisted of a solid-type tumour of 1 9 1 cm, with a white homogeneous surface on lamellation. The fragment was covered by normal mucosa. Histopathology
Fig. 1 a HRCT: axial view: round soft-tissue mass at the bottom of the right nasal cavity opacifying the left nasal vestibule. There is a normal linear cortication present. b 3D-CT reconstruction: impression
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demonstrated on the surface respiratory epithelium and squamous epithelium. Below the mucosa, lymphocytes and plasma cells were found. The nodule itself consisted of dilated venous vessels covered by flattened endothelial cells. These vessels are surrounded and separated from each other by spindle-shaped cells with a fibrous eosinophilic cytoplasm compatible with smooth muscle cells; sometimes these cells are more polygonal. The spindle-shaped cells and more polygonal cells are immunoreactive for smooth muscle actin (SMA), caldesmon (CALD) and desmin (DESM) to a lesser extent. The dilated venous vessels are covered with cells immunoreactive for CD 31 (also known as Platelet endothelial cell adhesion molecule: PECAM). No significant immunoreactivity was found to S 100. Some rare cells were immunoreactive for KI-67. According to the histopathology, the lesion was diagnosed as a vascular leiomyoma (Fig. 2).
Discussion A systematic literature review of articles published in English was performed using the PubMed database focussing on vascular leiomyomas limited to the nasal cavity excluding sinuses (Table 1). Of the 33 published cases of vascular leiomyomas within the nasal cavity, our case included, the mean age was 54 years with a median age of 50 years for women and 62 years for men. The age of the patients ranged from 24 to 86 years. Vivacqua also found a slightly different age between men and women [9]. Twenty-four tumours occurred in female patients and only 9 in male patients (ratio M/F 1/2.7). The size of the tumour ranged from 2 to 43 mm in diameter; most of them were less than 20 mm. The most affected nasal sites were the inferior turbinate, septum and vestibule.
and bone remodelling at the right processus alveolaris of the maxillary bone can be seen at the level of the tumour
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Fig. 2 Histopathology of the resected tumour in the right nasal cavity. a Hematoxylin–eosin staining (9400): the tumour consists of dilated venous vessels covered by flattened endothelial cells. These vessels are surrounded and separated from each other by spindleshaped cells with a fibrous eosinophilic cytoplasm compatible with
smooth muscle cells. b CD31 staining for endothelium (9400): the dilated venous vessels are covered with cells immunoreactive for CD 31. c Actin staining for smooth muscle cells (9200): the spindleshaped cells and more polygonal cells are immunoreactive for smooth muscle actin. d Caldesmon staining for smooth muscle cells (9400)
Leiomyoma has many variants. Histological classification by the World Health Organisation (WHO) divided the leiomyomas into three groups: leiomyoma, angioleiomyoma (vascular leiomyoma, angiomyoma) and epithelioid leiomyoma [3, 10, 11]. In 2002 the WHO defined angioleiomyoma as ‘‘a frequently painful, benign subcutaneous or deep dermal tumour composed of mature smooth muscle bundles which surround and intersect between vascular channels’’ [12]. So the vascular leiomyomas were classified to the group of ‘smooth muscle tumours’. However, according to the 2013 update, angioleiomyoma was reclassified under the ‘pericytic (perivascular) tumours’. This is because it shares morphological features with myopericytoma by showing perivascular concentric arrangement of smooth muscle cells [12]. The cause of angioleiomyoma is still uncertain. Three hypotheses have been proposed to explain the origin of smooth muscle tumours in the nasal cavity [13]. Firstly,
they could originate from aberrant undifferentiated mesenchyme; secondly they could arise from smooth muscle cells of the blood vessel walls and finally they might originate from arrector pili muscles around sweat glands in the nasal vestibule [14, 15]. Also hormonal imbalance, steroid therapy and trauma have been implicated as causative factors [16, 17]. The presence of progesterone receptor expression in sinonasal leiomyoma has been reported [18]. In the present case report, the patient had undergone a septoplasty that may also implicate an iatrogenic trauma to the nose. These tumours grow slowly and may persist for a long time [19]. According to the literature, the most common symptoms are nasal obstruction, epistaxis and facial pain (Table 1). They can also present with headache or acute sinusitis. The presence of chronic rhinosinusitis, like in our case, was not described in any other case and is possibly a coincidental finding.
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Eur Arch Otorhinolaryngol Table 1 Summary of previously reported cases of vascular leiomyomas in the nasal cavity Authors
Years
Gender
Age
Symptoms
Site
Treatment
Maeseka et al.
1966
F
49
Facial pain
Vestibule
Excision
Wolfowitz et al.
1973
F
42
Epistaxis
Inferior turbinate
Excision
Timiryaleev
1973
F
25
Epistaxis, nasal obstruction, headache
Septum
Excision
McCaffrey et al.
1978
F
76
Epistaxis, nasal obstruction
Inferior turbinate
Excision
Daisley
1987
F
32
Nasal obstruction, headache
Middle turbinate
Excision
Hanna et al.
1988
F
64
Epistaxis, nasal obstruction, facial pain
Inferior turbinate
Excision
Sawada
1990
F
41
Nasal mass
Vestibule
Excision
Ragbeer and Stone
1990
M
49
Epistaxis, facial pain
Nasal fossa floor
Excision
Khan et al.
1993
F
71
Nasal obstruction
Inferior turbinate
Excision
Ardekian et al.
1993
F
54
Epistaxis, nasal obstruction, facial pain
Nasal septum
Excision
Trott et al. Nall et al.
1994 1996
F F
43 43
Nasal obstruction, epistaxis Epistaxis, nasal obstruction, headache
Inferior turbinate Superior turbinate
Excision Embolization and excision
Melgarejo et al.
1997
F
62
Nasal obstruction
Inferior turbinate
Excision
Murono et al.
1998
F
69
Epistaxis
Inferior turbinate
Endoscopic excision
Bloom et al.
2001
F
50
Nasal obstruction, headache
Nasal septum
Endoscopic excision
Bloom et al.
2001
F
70
Asymptomatic
Nasal septum
Endoscopic excision
Osaki et al.
2002
M
67
Nasal obstruction
Nasal septum
Excision
Marioni et al.
2002
F
70
Nasal obstruction, epistaxis
Vestibule
Excision
Campelo et al.
2003
F
44
Epistaxis, nasal obstruction
Inferior turbinate
Endoscopic excision
Wang et al.
2004
M
70
Epistaxis
Nasal septum
Excision
Wang et al.
2004
M
62
Nasal obstruction
Vestibule
Excision
Wang et al.
2004
F
66
No symptoms
Inferior turbinate
Excision
Bel et al.
2005
F
50
Nasal obstruction, nasal discharge
Middle turbinate
Excision
Tsobanidou
2006
M
86
Nasal obstruction
Inferior turbinate
Excision
Tsobanidou
2006
F
63
Nasal obstruction, facial pain
Superior turbinate
Excision
Meher et al.
2007
F
24
Epistaxis
Middle turbinate
Endoscopic excision
Vafiadis et al. Michael et al.
2008 2009
M M
68 34
Nasal obstruction Nasal obstruction, epistaxis
Vestibule Inferior turbinate
Excision Endoscopic excision
Junior et al.
2010
F
62
Nasal obstruction, epistaxis and nasal pain
Nasal septum
Endoscopic excision
Arruda et al.
2014
F
49
Nasal obstruction
Inferior nasal concha
Excision
Hammedi et al.
2014
F
42
Nasal obstruction, epistaxis
Nasal septum
Excision
Varghese et al.
2015
M
40
Epistaxis
Inferior turbinate
Endoscopic excision
Present case
2015
M
54
Nasal obstruction, epistaxis
Nasal fossa floor
Excision
Histopathological differential diagnosis includes hemangioma, fibromyoma, angiofibroma, leiomyoblastoma, angiomyolipoma, vascular leiomyosarcoma [20]. In hemangiomas, the intervascular stroma does not have smooth muscle bundles as in an angioleiomyoma. Angiofibromas are tumours with proliferated thin walled staghorn vascular channels in a stroma containing round to stellate to spindle-shaped fibroblasts [21]. Malignant variants and recurrences of this neoplasm have been reported but are extremely rare. According to Hachisuga et al., only 0.3 % of 562 cases angioleiomyomas had local recurrence and none of them had malignant transformation [4]. Most of the tumours published were classified as benign. None showed mitosis or the presence of atypia, which may suggest malignant behaviour. It appears that the absence of mitosis is the most useful histologic indicator of benign lesions.
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Moreover, size, infiltration, mitotic index and bone erosion in significant sites are valuable diagnostic aids for these tumours [6]. In the present patient, not bony erosion but bone remodelling was found at the base of the maxillary bone probably due to longstanding pressure of the tumour on the bone. In order to diagnose a vascular leiomyoma, a great deal of diagnostic alertness is needed. Imaging by both CT and MRI of these tumours is not specific [22]. However, MRI and CT can provide information about the extent and the anatomical position. 3D CT scan preoperatively can show the delineation of the tumour very accurately helping to establish the therapeutic plan for removal. The definite diagnosis is set by histopathological examination. The treatment of choice for these lesions, as in other anatomical regions, is complete surgical excision, which can
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mostly be accomplished because the mass can be removed along with a surrounding rim of normal mucosa. There are no reports of recurrence after total excision [10, 20, 23]. Potential recurrence after incomplete resection has been shown [10]: so complete excision is necessary in order to guarantee an effective and definitive treatment. The choice of the surgical approach, by lateral rhinotomy, subgingival incision or by nasosinusal endoscopic surgery, will depend on the tumour localization and extension, as well as the need for a better bleeding control [10, 20, 22]. Surgical resection is necessary if the lesions become symptomatic or if there is any doubt concerning the nature of the lesion. However, otolaryngologists must be aware of the risk of delaying surgical intervention. Nicolai et al. reported a case of a vascular leiomyoma of the nasal cavity extending into the nasopharynx and the anterior cranial fossa. Craniofacial resection was needed to eradicate the vascular leiomyoma [24].
Conclusion Vascular leiomyoma of the nasal cavity is an extremely rare benign tumour. It is more prevalent in females and middle aged patients and the most affected sites are the nasal septum and the inferior turbinates. Vascular leiomyomas are more frequent in the fourth, fifth, and sixth decades of life, and more than 80 % of vascular leiomyomas are less than 2 cm in diameter. They arise most commonly in the deep layers of the dermis or in the subcutaneous tissues. The primary symptoms are nasal obstruction, epistaxis and facial pain. Although the occurrence in the nasal cavity is very rare, otorhinolaryngologists should be aware of the possible existence of angioleiomyoma in the nasal cavity. A preoperative 3D CT scan has an added value in the diagnosis and treatment of these tumours. Treatment is based on complete surgical excision. Delay of the surgical eradication must be avoided. The prognosis is very good. Compliance with ethical standards Funding This study is not funded. Conflict of interest All authors declare that they have no conflict of interest. Ethical approval This article does not contain any studies with human participants or animals performed by any of the authors.
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